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[Recombinant human activated protein C in the treatment of children with meningococcal purpura fulminans]. 2004

F Martinón-Torres, and J María Iglesias Meleiro, and M Fernández Sanmartín, and A Rodríguez Núñez, and J María Martinón Sánchez
Servicio de Críticos, Intermedios y Urgencias Pediátricas, Departamento de Pediatría, Hospital Clínico Universitario de Santiago de Compostela, Universidad de Santiago de Compostela, Santiago de Compostela, Spain. fedemartinon@movistar.com

Meningococcal purpura fulminans (MPF) produces high mortality and morbidity, despite appropriate standard therapy. Administration of recombinant human activated protein C (rhAPC) has been successfully applied in adults with MPF and pediatric studies are under way. We report three pediatric patients with MPF treated with rhAPC as compassionate therapy. In two of these patients, positive clinical and laboratory effects were observed and both children achieved full recovery. The remaining patient died after 36 hours from refractory multiorgan failure. No rhAPC-related adverse effects were detected. The reported cases highlight the usefulness of rhAPC in children with MPF at least as a rescue compassionate treatment. Further clinical trials are needed to better delineate its efficacy and administration schedule in children.

UI MeSH Term Description Entries
D008297 Male Males
D011486 Protein C A vitamin-K dependent zymogen present in the blood, which, upon activation by thrombin and thrombomodulin exerts anticoagulant properties by inactivating factors Va and VIIIa at the rate-limiting steps of thrombin formation.
D011994 Recombinant Proteins Proteins prepared by recombinant DNA technology. Biosynthetic Protein,Biosynthetic Proteins,DNA Recombinant Proteins,Recombinant Protein,Proteins, Biosynthetic,Proteins, Recombinant DNA,DNA Proteins, Recombinant,Protein, Biosynthetic,Protein, Recombinant,Proteins, DNA Recombinant,Proteins, Recombinant,Recombinant DNA Proteins,Recombinant Proteins, DNA
D001779 Blood Coagulation Factors Endogenous substances, usually proteins, that are involved in the blood coagulation process. Clotting Factor,Coagulation Factors,Blood Coagulation Factor,Clotting Factors,Coagulation Factor,Coagulation Factor, Blood,Coagulation Factors, Blood,Factor, Coagulation,Factors, Coagulation,Factor, Blood Coagulation,Factor, Clotting,Factors, Blood Coagulation,Factors, Clotting
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D005260 Female Females
D005343 Fibrinolytic Agents Fibrinolysin or agents that convert plasminogen to FIBRINOLYSIN. Antithrombic Drug,Antithrombotic Agent,Antithrombotic Agents,Fibrinolytic Agent,Fibrinolytic Drug,Thrombolytic Agent,Thrombolytic Agents,Thrombolytic Drug,Antithrombic Drugs,Fibrinolytic Drugs,Thrombolytic Drugs,Agent, Antithrombotic,Agent, Fibrinolytic,Agent, Thrombolytic,Agents, Antithrombotic,Drug, Antithrombic,Drug, Fibrinolytic,Drug, Thrombolytic,Drugs, Antithrombic
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293 Adolescent A person 13 to 18 years of age. Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D014884 Waterhouse-Friderichsen Syndrome A condition of HEMORRHAGE and NECROSIS of the ADRENAL GLAND. It is characterized by rapidly developing ADRENAL INSUFFICIENCY; HYPOTENSION; and widespread cutaneous PURPURA. Fulminant Meningococcal Sepsis with Adrenal Apoplexy,Meningococcal Hemorrhagic Adrenalitis,Purpura Fulminans (Waterhouse-Friderichsen Syndrome),Meningococcal Waterhouse-Friderichsen Syndrome,Waterhouse-Friederichsen Syndrome,Adrenalitides, Meningococcal Hemorrhagic,Adrenalitis, Meningococcal Hemorrhagic,Hemorrhagic Adrenalitides, Meningococcal,Hemorrhagic Adrenalitis, Meningococcal,Meningococcal Hemorrhagic Adrenalitides,Meningococcal Waterhouse Friderichsen Syndrome,Syndrome, Meningococcal Waterhouse-Friderichsen,Waterhouse Friderichsen Syndrome,Waterhouse Friederichsen Syndrome,Waterhouse-Friderichsen Syndrome, Meningococcal

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